Laparoscopic cholecystectomy and postoperative pain control in a patient with chronic non-spherocytic hemolytic anemia from glucose-6-phosphate dehydrogenase deficiency

Watanabe, Megumi; Kashiwadate, Toshiaki; Hagiwara, Motohisa; Hashizume, Eiji

doi:10.1016/j.jclinane.2018.10.029

Cited by 4 publications

(1 citation statement)

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“…However, the development of a gene therapy for G6PD def presents a major challenge; the cost per betibeglogene autotemcel treatment course is set at US $1.8 million with high costs common in gene therapy [114]. Although rare, G6PD def Class I variants have a severe phenotype, impacting the patient's quality of life [115,116]. Early studies found retroviral transduction of the human G6PD gene into mouse and human hematopoietic stem cells resulted in stable lifelong expression of human G6PD in primary and secondary bone marrow transplant recipient mice, increasing G6PD activity twofold [117], suggesting gene therapy for the most severe variants of G6PD def is warranted.…”

Section: Key Figurementioning

confidence: 99%

Treatment strategies for glucose-6-phosphate dehydrogenase deficiency: past and future perspectives

Garcia

Koperniku

Ferreira

et al. 2021

Trends in Pharmacological Sciences

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Glucose-6-phosphate dehydrogenase (G6PD) maintains redox balance in a variety of cell types and is essential for erythrocyte resistance to oxidative stress. G6PD deficiency, caused by mutations in the G6PD gene, is present in ~400 million people worldwide, and can cause acute hemolytic anemia. Currently, there are no therapeutics for G6PD deficiency. We discuss the role of G6PD in hemolytic and nonhemolytic disorders, treatment strategies attempted over the years, and potential reasons for their failure. We also discuss potential pharmacological pathways, including glutathione (GSH) metabolism, compensatory NADPH production routes, transcriptional upregulation of the G6PD gene, highlighting potential drug targets. The needs and opportunities described here may motivate the development of a therapeutic for hematological and other chronic diseases associated with G6PD deficiency. G6PD deficiency as a hematological disorderGlucose-6-phosphate dehydrogenase (G6PD; see Glossary) regulates glycolytic flux through the pentose phosphate pathway (PPP) and plays a central role in redox homeostasis; it produces NADPH, an essential cofactor for glutathione (GSH) regeneration. G6PD is a major source of NADPH and loss of G6PD function is detrimental in erythrocytes; it causes G6PD deficiency (G6PD def ), a potentially hemolytic disorder [1]. HighlightsGlucose-6-phosphate dehydrogenase (G6PD) deficiency is better known as a hematological disorder. However, G6PD may play a role in other chronic disorders such as diabetes, cardiovascular disease, viral infectivity and complications, and neurodegeneration.

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